Abstract:
:Niemann-Pick C (NPC) disease is a progressive neurological disorder in which cholesterol, gangliosides and bis-monoacylglycerol phosphate accumulate in late endosomes/lysosomes. This disease is caused by mutations in either the NPC1 or NPC2 gene. NPC1 and NPC2 are involved in egress of lipids, particularly cholesterol, from late endosomes/lysosomes but the precise functions of these proteins are not clear. An important question regarding the function of NPC proteins is: why do mutations in these ubiquitously expressed proteins have such dire consequences in the brain? This review summarizes the roles of NPC proteins in lipid homeostasis particularly in the central nervous system.
journal_name
FEBS Lettjournal_title
FEBS lettersauthors
Vance JEdoi
10.1016/j.febslet.2006.06.008subject
Has Abstractpub_date
2006-10-09 00:00:00pages
5518-24issue
23eissn
0014-5793issn
1873-3468pii
S0014-5793(06)00710-1journal_volume
580pub_type
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